Article
Transgenic mice overexpressing mutant PRKAG2 define the cause of Wolff-Parkinson-White syndrome in glycogen storage cardiomyopathy.
Circulation - 10 Jun 2003
Arad Michael, Moskowitz Ivan P, Patel Vickas V, Ahmad Ferhaan, Perez-Atayde Antonio R, Sawyer Douglas B, Walter Mark, Li Guo H, Burgon Patrick G, Maguire Colin T, Stapleton David, Schmitt Joachim P, Guo X X, Pizard Anne, Kupershmidt Sabina, Roden Dan M, Berul Charles I, Seidman Christine E, Seidman J G
Abstract excerpt
BACKGROUND: Mutations in the gamma2 subunit (PRKAG2) of AMP-activated protein kinase produce an unusual human cardiomyopathy characterized by ventricular hypertrophy and electrophysiological abnormalities: Wolff-Parkinson-White syndrome (WPW) and progressive degenerative conduction system disease. Pathological examinations of affected human hearts reveal vacuoles containing amylopectin, a glycogen-related...
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